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Effective treatment of ligneous conjunctivitis with topical plasminogen
Patrick Watts1, Palanisamy Suresh, Eedy Mezer
1Department of Ophthalmology, The Hospital for Sick Children and Toronto Western Hospital, Toronto, Ontario, Canada.
Insights
Ligneous conjunctivitis, a chronic membranous condition, is caused by plasminogen deficiency. Topical plasminogen treatment effectively resolved symptoms in three pediatric cases, preventing recurrence.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Ligneous conjunctivitis is a rare, chronic inflammatory condition characterized by thick, woody-like deposits on the conjunctiva.
- The underlying etiology has been identified as a deficiency in plasminogen, a key protein in the fibrinolytic system.
Observation:
- This case series presents three pediatric patients diagnosed with ligneous conjunctivitis.
- Two patients were female Caucasians (5 years old) and one was a male of North African descent (18 months old).
- Clinical features included recurrent membranous conjunctivitis, sometimes associated with systemic conditions like bronchiolitis and gingival hyperplasia.
Findings:
- Laboratory results confirmed significantly low plasminogen levels in all three patients, consistent with plasminogen deficiency.
- Following surgical excision of conjunctival membranes and the institution of topical plasminogen drops, all patients showed a positive response.
- No recurrence of conjunctivitis was observed in any patient after more than 12 months of follow-up.
Implications:
- Topical plasminogen administration, derived from fresh frozen plasma, shows promise as a definitive treatment for ligneous conjunctivitis.
- Further research is needed to optimize plasminogen delivery methods for this chronic condition.
- Understanding the genetic basis of plasminogen deficiency is crucial for managing this rare disorder.
Purpose:
The etiology of ligneous conjunctivitis is now known to be due to an underlying type 1 plasminogen deficiency. We hereby report the clinical features of three cases and their response to topically administered plasminogen.
Design:
Observational case series.
Methods:
Two Caucasian females aged 5 years and an 18-month male of north African descent presented with a membranous conjunctivitis, which recurred after surgical excision. Case 1 presented before the association with plasminogen deficiency was known with a bilateral chronic membranous mucopurulent conjunctivitis from the age of 14 months associated with bronchiolitis and gingival hyperplasia. A diagnosis of ligneous conjunctivitis was entertained and a number of drops were instituted. At the age of 4 years plasminogen levels were ordered. Case 2 presented at the age of 4 years with a unilateral chronic membranous conjunctivitis. Plasminogen levels were requested as soon as a diagnosis of ligneous conjunctivitis was suspected. Case 3 was born with congenital hydrocephalus. Conjunctivitis was treated with antibiotics from the age of 1 month. He presented to the eye clinic at the age of 5 months when a clinical diagnosis of ligneous conjunctivitis was entertained and treated with a number of medications. Plasminogen levels were available at 9 months of age.
Results:
The two female patients returned plasminogen levels of 0.25 U/ml and 0.3 U/ml, well below the normal level of 0.7-1.0 U/ml. Functional plasminogen levels in the male infant were not recordable with plasminogen antigen levels of 0.125 U/ml (normal range, 0.52-1.82). All cases have responded well to excision of the membranes and institution of topical plasminogen drops. There has been no recurrence with more than 12 months' follow-up.
Conclusions:
With the knowledge of the etiology of ligneous conjunctivitis, efforts are underway to identify the best method of delivery of plasminogen. Topical plasminogen concentrate from fresh frozen plasma holds promise as the definitive treatment for this chronic membranous conjunctivitis